NPC1 antibody (clone 4H2), anti-human

NPC1 antibody (clone 4H2), anti-human

€435.00
In stock
SKU
AC-AT3083a
Catalog Number: AC-AT3083a
Size: 100 µg
Isotype: mouse IgG2a Kappa
Applications: WB, E
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Background:
This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

Other Names:
Niemann-Pick C1 protein, NPC1

Antigen Type:
Recombinant Protein

Gene Name: NPC1 (HGNC:7897)
Gene ID: 4864
NCBI Accession: NP_000262.2
Primary Accession: O15118
Other Accession: BC063302
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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